Prevalence of sleep-talking in childhood.

This report is on the prevalence of somniloquy, as revealed by a populational survey of 2,022 schoolchildren aged from 3 to 10 years. Data were collected by means of a multiple choice questionnaire aimed at the investigation of sleep characteristics (sleep characteristics questionnaire-SCQ) and submitted to statistical analysis. The objective was to detect the prevalence of sleep-talk and influence of age, sex and socioeconomic class on its frequency. About half the children of all ages presented somniloquy at least once a year, but less than 10% presented it everyday. No clearmarked correlation with age, sex or socioeconomic class was found. These results do not confirm the generally accepted notion that there is a higher prevalence in the beginning of the first decade of life and suggest also the necessity of further quantitative and objective assessment of this phenomenon.

Evaluation of flurazepam and placebo on sleep disorders in childhood.

The clinically observed results in 40 patients, from 1 to 15 years old, presenting sleep disturbances, in a comparative and statistically approached study of flurazepam 15mg daily against placebo, are reported. Placebo was administered, followed by the drug, during 14 days each. The chief complaints were sleepwalking, sleep-talking, sleep terror, sleep-related bruxism, sleep-related headbanging, insomnia and excessive movements during sleep. A significant effect of flurazepam on sleepwalking, sleep-talking, bruxism, sleep terror and excessive movement during sleep, was observed. The insomniac headbanging patients were not enough for statistical analysis. Flurazepam side effects were excessive drowsiness during daytime in 3 cases; irritability, 3 cases; nausea and vomiting, 2 cases, and were not correlated with age. Placebo side effects were similar, except for nausea and vomiting which were not observed. It was necessary to discontinue flurazepam in 2 cases, because of excessive drowsiness during daytime, which did not improve when reducing the dose.

[Atypical Sturge-Weber disease associated with metameric angiomatosis. Case report and review of the literature]. / Doença de Sturge-Weber atípica associada a angiomatose metamérica. Registro de um caso e revisão da literatura.

Possibly the first case of Sturge-Weber disease associated with metameric limbs and trunk cutaneous hemangioma is reported. Roentnographic findings including contrast studies and computerized axial tomography are presented and discussed. Comments are made upon the relations between neurocutaneous angiomatosis.

[Eosinophilic granuloma of the cervical spine with atypical neurological, cerebrospinal fluid and radiological manifestations]. / Granuloma eosinofilo da coluna cervical com manifestacão neurologica, liquorica eradiologica atipica

A case of eosinophilic granuloma of the cervical spine in a ten year-old white boy is reported. The patient complained of posterior cervical pain at the level of C5-C6 and right hemiparesis that progressed to mixed tetraparesis (lower motor neuron in upper limbs and upper motor neuron in lower limbs). Examination of the cerebrospinal fluid (CSF) disclosed an inflammatory reaction; radiologic studies of the cervical spine showed an irregular aspect with demineralization at the base of spinous process of C6. The patient was treatet intermitently with corticosteriods (prednisone); there was an improvement of neurologic symptoms and the examination of the CSF was normal within a few days, each time he took the drug. Neurologic symptons and abnormalities of the CSF recurred after discontinuation of corticotherapy. Examination of a fragment of the spinous process and neighboring tissues of C6, taken by biopsy, disclosed eosinophilic granuloma. Radiotherapy was then indicated, plus corticosteroids. There was remission of all neurologic symptoms and signs. The unusual features of the case are discussed in relation to others reported in the literature.

[Teratoma of the 4th ventricle and precocious puberty. Case report]. / Teratoma do IV ventrículo e puberdade precoce. Registro de um caso.

A case of precocious puberty and diabetes insipidus in a 7 years old boy due to a malignant teratoma in the IVth ventricle is reported. The tumor had grown into the IIIrd and lateral ventricles as found on the necropsy. The original site of the tumor and the possible physiopathological mechanisms for the precocious puberty are discussed.

[Sjögren-Larsson syndrome. Clinical and laboratory study of 2 cases]. / Síndrome de Sjögren-Larsson. Estudo clínico e laboratorial de dois casos.

Two cases of Sjögren-Larsson syndrome are reported. In analysing the clinical symptoms and laboratory data the authors established the diagnosis of this rare entity which has an autosomal recessive kind of inheritance.

[Menkes syndrome: review of the pathogenesis apropos of a clinico-pathological case]. / Síndrome de Menkes: revisão da patogenia a propósito de um caso anátomo-clínico.

The authors report a case of Menkes' syndrome, probably the first one described in Brazil. The patient, a 15-month-old boy, showed pili torti, early progressive psychomotor deterioration and seizures. Serum levels of ceruloplasmin and copper were very low. Neuroradiological and roentgenological examinations revealed diffuse cerebral atrophy, arterial changes and bone abnormalities. At the post-mortem examination the more consistent findings were cerebral atrophy, neuronal loss in the thalamus and above all cerebellar cortical lesions. The disease has a sex-linked recessive inheritance and is believed to be caused by an inborn error of copper metabolism, perhaps subordinated to changes of proteins which carry copper to different tissues. The relevant literature in relation to the pathogenesis is reviewed.

Unusual subarachnoid metastasis of an intracranial chordoma in infancy.

The authors report a case of large clivus chordoma in a 61/2-year-old child with multiple subarachnoid metastatic dissemination over the cerebellar hemispheres and spinal cord.